Pagets Disease:

Ethiology:

Pathology:

Who?:

Clinical:

Problem:

cottonwool radiopaque areas, resembles fibrous displasia, hypercementosis

Lab:

Ethilogy: unknown, probably virus

pathology: ^ ostoclastic resoprt, abnormal osteobl deposition,

Who?: >40y/o

Clinical: stooped posture, enlarge skull and face bone, deformity of spine and long bones. bowing of lower extremities.

Problem: narrowing of skill foramina, cause cranial nerve dysfunction. dentures dont fit due to enlargement of alveolar ridge. Space btwn teeth

Lab: increases serum alkaline phosphatase

Pagets Disease

Tx and prognonsis:

calcitonin and diphosphonates inhibit osteoclastic resorbtion

osteosarcoma

Hyperthyroidism

Primary: parathyroid hyperplasia, adenoma, carcinoma-> increased PTH

Secondary: kidney disease >> decreased CA resorption and hypocalcemia>> increased PTH

CLINICAL:

LAB:

Tx:

Clinical: kidney stones and gastrointestinal ulcers; decreased bone denity> "ground glass", loss of trabeculation, lamina dura, cortical plates

giant cell tumors

LAB: primary:^ serum Ca and PTH

secondary: dec or normal ca, inc PTH

TX: Primary: PTG exscision

Secondary: dyalisis or renal transplant

Osteogenesis Imperfecta: genetic

what:

clinic:

what: defective cross-linking of collage, and osteoid

clinic: fragile, brittle bone

congenital: multiple fractures in utero, die after birth

tarda: milder form w/ normal lifespan

large skull, thin, with prominet frontal bones.

kyphosis, scoliosis, hyperextensible joints

Osteogenesis Imperfecta

Eyes:

Ears:

Heart:

prognosis:

Eyes: blue sclerae

ears: hearing loss

Heart: increased incidense of mitral valve prolapse requiring prophylactic antibiotics

prognosis: varies from death at brith to normal. Decreased fractures with age.

Dentinogenesis Imperfecta

teeth:

crowns,neck and root:

pulp chamber and canals

enamel:

Dentin Only

Teeth: primary teeth are more severly affetcted than permanent dentition

appear translucent and blue
C,N,R: bublous crowns, thin necks and roots that fracture easily

pulp chambers and canales ar enlarged or obliterated with abnormal dentin

enamel is normal, but fractures easily - rapid attrition

Achondroplasia:

Pth:

Cx:

TX/PX

Pth: defective calcification of cartilage in epiphyseal plate; decreased log bone=dwarfism

Cx: stillborn or die short after birth; short thock arms and bowed legs.

Short wide skull, lordosis, retruded maxilla

TX/PX: no tx, if pt survives earloy childhood then normal lifespan

Gardner's Syndrome: GI sarcoma

Familial Multi ple Polyposis of colon: can have carcinoma but no osteomas

peutz-jeghers Syndrome: polyps have low malignant potential

Turcots syndrome: colonic polyps and brain tumors

Gardners Syndrome" Genetic

**3/4 of pts have dental abnormalities or osteomas; cancer

Clinic:

bone:

skin:

Teeth:

Cx: polyps (adenomas) of colon and rectum, cancer

bone: multiple osteomas of bone (preced polyps)

in calvarium and facial skeleton

skin: epiderm cysts and fibrous tumors of skin

Teeth: unerupted permanent and supernumerary teeth

Gardner's Syndrome

Tx and Px

Tx an dPX:

polyps: should be periodically evaluated, colectomy

Px: depends on intestinal carcinoma

Cherubism (genetic

Age: 

Cx:

Radiographic:

teeth:

Age: 3-4yrs

Cx: progressive non-tender swealling of jaws

regional lymphadenopathy is common

Rx: bilateral radiolucent lesions> expansion and perforation of cortices

teeth: multiple unerupted teeth displased by lesions; premature exfoliation of primary teeth and unerupted permanent teeth

Cherubism

Tx and PX

Tx and pX

lesions stabilize at puberty

diff to remove lesions

surg recontpouring for esthetics

cranopfacial Dysostosis (Crouon disease)

GENETIC = early closure of sutures

CX:

eyes:

Oral:

Apert Syndrome:

Cx: froglike face due to midface hypoplasia and exophalmos

Craniofacila: hypoplastic maxilla, parrot beak nose

eyes: exophthalmos, interpupillary distance, blindness due to nerve damage

Oral: high palate, cleft; hypoplastic maxilla, malocclusion

Apert Syndrome: craniofacial dysostosis with webbing between adhacent fingers and toes (syndactyly)

Craniofacial Dysostosis (Crouzon Disease)

Tx And PX

TX and PX

Craniectomy early in life to provide space for the brain

ortho and orthognathic surgery

ComplicationsL impaired hearing, speech, vision, mental retard

may have normal lifespan

Cleidocranial Dysplasia: GENETIC

Skull:

shoulder girdle:

Oral:

teeth:

Skull: Open Fontanels or late closure

Open Sutures>>Wormian bones

Brachycephalic skull

PROMINENT frontal, parietal and occipital bones

Shoulder girdle: aplasia, hypoplasia of clavicles.

Hypermob of shldr
Oral: high platae, cleft, hypoplastic maxilla and sinuses

teeth: ret of primary teeth, succ fail to erupt due to lack of cellualar cementum

***Multiple unerupted supernumerary teeth,

severe mal occlusion

Mandibulofacial Dysostosis

(Treacher Collins Syndrome)

GENETIC

Face:

eyes:

ears:

oral:

Face: convex facial profile> down sloping palpebral fissures, hypoplasia mandible, fishlike mouth

Eyes: fissure (coloboma) of lower eyelid; no eyelashes

ears: deformed pinna, conductive hearing loss

Oral: hypo mand, high plalate, cleft, malocclusion

Mandibulofacial Dysostossis

(treachir collins syndrome)

Tx and PX

Tx and PX

surgery fpor eyes and ears, ortho, and orthognathic surgery

Normal lifespam

Marfans Syndrome

GENETIC

What:

Fingers:

Joints:

Heart:

oral

eyes:

what: defective crosslinking of collage, increase length of tubular bones and long, thin extremities (loincoln)

fingers: arachnodactyly (spiderlike)

jpinmts: hyperextensible, dyslocations

heart: aortic aneurisma, valve prolapse

oral; high palate

eyes: dyslocation of lens and myopia

Marfans Syndrome

Tx and PX

Tx and PX

prophylactic abx,

guarded px due to cardiovascular problems

Neurofribromatosis

(Von R - skin)

skin:

skin: multiple neurofibromas:

- well circumscribed, exophytic, deep fissures

- cafe au lait spots: melanotic macules >1.5 cm

freckles in axilla

Nurofibromatosis:

oral

Body:

TX and PX

oral: oral neurofibromas in 7-20%, well circumscirbed or difused

Macroglosia

soft tissue sarcoma occur in <10%

Body: bony lesions, CNS lesions

TX PX: cant remove all fibromas, cosmetic problem, sarcomas poor px

Basal Cell Nevus Syndroms

Skin:

when:

bone:

skin: multpl basal cell carcinoma, pits on hand and feet, hyperkeratosis, cyst and benign tumors

when: early in life,

Tx: req agressive surgery

bone: keratocysts of jaws that interfere with tooth eruption

Bifid ribs

Vertebral: spina bifida, scoliosis, short fingers

Basal Cell Nevus Syndrome

Neurologic:

Ophthalmolgic:

Neurologic: calcificatipon of dura mater, meduloblastoma

Ophth: hypertelorism, dystopia canthorum: malposition of canthu

internal strabismus

blindness

Basal Cell Nevus Syndrome:

Tx and Px

Tx and PX

Basal cell carcinoma tx by Mohs surgery

-keratocyst surgically removed

Px: normal life spam

Ectodermal Dysplasia

2 major types:

Anhidrotic

Hidrotic

Ectodermal dysplasia

Anhidrotic:

Hidrotic

facial:

teeth

anhidrotic: partial or complete absence of sweat glands, seb glands, and hair folicules

facial: prominent frontal bone; midface hypoplasia and saddle nose

teeth: hypo, anodontia, hypo alveolar ridge, coneshaped or unerupted teeth

Ectodermal Dysplasia

Tx and PX

TX and PX

Cool envirnments, lubricnt to dyr skin, dental prosthesis

PX: normal lifespan

Gardners Syndrome

Cleidocranial dysplasia

Ectodermal dysplasia

cherubism

Peutz-Jeghers Syndrome

Skin and oral mucosa:

Intestinal polyps

skin and oral mucosa: melanotic macules of oral mucosa; freckles on face around eyes, nose, lips

Intestinal polyps: mainly small intestine, hamrtomas with low malignant potential

Papillon-Lefevre Syndrome

(juvenile Periodontosis)

Mouth:

skin:

mouth: severe alveolar bone loss, gingivitis, deep pockets, premature loss of teeth

skin: hyperkeratosis of palsm and feet

Multiple Endocrine Neoplasia, Type III

(MEN III)

two main problems:

-medullary carcinoma of thyroid

> can meta and cause death from parafollicular cells

-pheochromocytoma of adrenal medulla

>>benign neoplasm, catecholamines causing hypertension

**Oral mucosal neuroma may be first sign of syndrome (lips tongue and buccal mucosa, dont recur after excision)

Red Blood Cell Disorders:

Anemia> blood loss, decrased prod, hemolytic (inc destr)

what?

lab test

SX

Decreased o2 carrying capacity

Lab test:

RBC 4-6x106/ul

Hb 12-18g/dl

Hct 37-54%

Sx: wakness, fatigue,lightheadm headache, pallor, jaundice, rapid pulse, concave fingernails, skin atrophy

Iron Deficiency Anemia

Head and Neck

head and neck:

burning tongu, erythema, atrophyt filiform papi;la, tast alteration, lip fissure, may candidosis

pernicious anemia

what?

samea as iron anemia sx

impaired vB12 absoprtion, lack of intrinsic factor, autoimmune.

Symmetric paresthesia- tingling or numbness of extremities

Decreased Production: Def Hemoglobin Synth

1)Irond deff anemia

- Plummer-Vison Syndrome

2) Aplastic Anemia

northern erupe wm 30-50yrs

5-50% risk of squamous cell carcinoma

2) precursor cell failure to produce all blood cell types

>>gingival blreeding, mucosal petichiae, gingival hyperplasia, mucosal ulcers, purpura

Increased Destruction:

1) Hemolytic anemia (avoid infections, hypothermia, hypoxia, acidosis, dehydration)

2) Sickle Cell Anemia- short app, simple, prev, Epi only for surgery, avoid barbiturates and strong narcotics. Valium OK

1) shortened l RBC life span, accumulation of products of hgb catabolism, marked increased in erythropoiesis. JAUDICE< SPLENOMEGALY

2) reduced trabeculation, hair on end appearance clvaria, delayed eruption, dental hypoplasia, ischemic necrosis, neuropathy

Bleeding disorders

b/c hered, illness, medications

common features

Common Features of Anemia

(7)

Weakness

Fatigue

Light head

headache

pallor/jaundice

inc hr

skin atrophy

Bleeding Disorders:

2

Hereditary Hemorrhagic Telangiectasia

Thrombocytopenia

Platelet Dysfunction

Hereditary Hemorragic Telangiectasia:

Cx:

Head and neck:

Mgt:

Cx: Tortous, thin-walled, dialted blood vessels

GI bleeding . anemia

predisposed to brain abscesses

Head and neck: blanching red mucosal papules of tongue, lips and nose (+nosebleeds)

Mgt: Abx prohy, hemostasis with direct compression

Platelets Labs:

Normal count:

Low:

Post-Traumatic bleeding:

Spontaneous bleeding:

***Petechia, purpura***

150k-300k/ul

<100k/ul

<=50k/ul

<=30k/u;

Decreased plate;et Production

(4)

1. Stem Cell Problems: aplastic anemia; bone marrow infiltration by malignancy (leuk, meta)

2. Defective DNA Synthesis: Megaloblastic anemia

3. Drug Induced: EtOH, thiazides, cytotoxic drugs

4. Infections: viral

Acquired Platelet Dysfunction:

(2)

*azotemia:

1. Renal failure; azotemia=excess urea, creatinine in the blood

2. Asprin and NSAID's: interfere w/ plat aggr, inc oozing

Clotting Factor Deficiencies:

Hereditary:

Acquired:

:hemophilias

: more common

If taking Anticoagulants (coumarins)

check for PT (prothrombin time):

Minor oral surgery measures:

INR 2.0-2.5 local measures

Not safe: >= 3.0

Target upt o 4.5 for mechanical prosthetic valve

2. gelatin sponge, sutures, tranexamic acid

Neutrophils:

disease:

engulf and digest pathogens; recruit other cells.

extracellular bacteria

2. agranulocytosis

Agranulocutysis:

what:

mouth:

**Comparable to ANUG (diff by biopsy)**

lack of neutrophils.

Deep, punched out ulcerations og buccal and gingival.

Neutropenia:

what:

Drug Induced:

Infection Induced

Secondary to what?:

nutrophil<=1500/ul

 Drug induced: chemo agents abxs, phenothiazines, diuretics, tranquilizers.

infection indiced: viral hp A,B; rubela, measles, varicella, HIV, typhoid, TB

 2. Secondary to one marrow destruction by malgnancies, osteporosis

neutropenia due to bacterial infections:

gingival ulcerations often w/o erythema

biopsy

blood test (CBC with differential, repeats)

Discontinue drug

May require culture

Abx tx

Recombinan human granulopoietic factors

1. dRUG INDUCED NEUTROPENIA

2. Infection Induced Neutropenia

1. Chemotherapeutic agents, abx, phenothiazines, diuretics, tranquilizers

2.viral hepatitis A and B, rubella, measles, varicella, HIV, thyphoid, TB, etc.

Oral manifestations of HIV

common:

less common:

common:

lymphadenopathy; CANDIDOSIS,

KAPOSIS SARCOMA (looks like a bruise or big on palate)

lesscommon:

HPV, histoplasmosis, lymphoma

Reactive WBC Proliferations:

Lymphoid hyperplasia

chronic:

confused w/ lypoma(shallow)

(pink to red if deep)

enlargement of any lymphoid tissue in response to antigens

chronic: enlarged, firm, rubbery, -+ tender

intraoral aggregates

***suspicious for neoplasia:

asymetric non-tender enlargement

1. any cell type ;or its precursor

2. lymphoma

3. leukemia

neoplastic disorder of blood cell.

tranformed RBC

by genetic and env, inmuno deff, Benzene, radiadion, viruses

Classification of leukemia:

1. Maturity: acute, chronic

2: Cell Type:

1. acute: very imature leokocutes(blasts)

chronic: relatively mature leukocytes

2. cell type: lymphoid, myelogenous

Acute leukemia:

what:

ALL

AML

rapid replacement of bone marrow by immature neoplastic cells >> in anemia, bleeding prb, and infections

ALL-children

AML: both

Systemic Acute Leukemia

myeloid:

lymphoblastic:

Abrupt onset:

anemia, bleeding disorder, infections, bone pain, lymphadenopathy, hepato- and splenomegaly

CNS: vomiting, headache, nerve palsies

candidosdis and herpes simplex

a. gingival inv (enlrgement and ulceration)

       b. lymphoid tissue inv

Acute leukemia

tx and px

tx: chemotherapy; bone marrow transplant; transfusions, abx

px: ALL: good 50-70% cure, adult 20%

AML: poor for both children and adult

(1-2yr survival) 10-30% survival

Chronic Leukemia:

CML

CLL (bad Herpes Simplex)

fatigue, weakness, weight loss, anorexia, hepatomegally, lymphadenopathy

-anemia, thromocytopenia (mild)

infections more problematic at later stages

may be asymptomatic for a while

Lymphoma

2 major categories

neoplastic disprder of WBC; malignant neoplasm of lymphocytes, histiocytes and their precursors

Hodgkin Disease (lymphoma)

Non-Hodgkin Lyphoma (NHL)

Hodgkin disease

tx and px

lymphoma; comon cancer of young adults (15-35ys)

 mainly involves lymph nodes cervical and supraclavicular

tx: local rad for stg I,II; chemo for III, IV

PX: 5yr I,II >90%; III 60-70%; IV 50%

Non-Hodgkin Lymphoma

nodal or extra nodal

oral cavity

WALDEYERS RING

Nodal: non-tender, slowly enlarging, initially freely movable in CERVICAL

single to multiple fixed masses wich feel conected

OC: ST: non tender, ill defined, swealling; erythema nad ulceration

>>asymetric, progessove enalrgements

extranodal Non-hodgkin

Bone:

advanced:

vague pain or discomfort, paresthesia, ill-defined radiolucency or xray

CAN displase and perforate bone cortex and cause soft tissue swealling

Advanced: malaise, fever, sweating, weight loss

biopsy for Dx

Staging

Cemo and or rad

Langerhans Cell Disease

(Histiocytosis X)

proliferatio of langerhans cells accompained by eosinophil. lymphocytes, plasma cells

MAY NOT BE A NEOPLASTIC DISEASE

1. one or more bone lesions

2. disseminated disease w/ skin, bone and internal organ involvement

**1/2pts <10y/o